Multifocal motor neuropathy and asymptomatic Hashimoto's thyroiditis: first report of an association

Motor neuropathy with multifocal conduction blocks represents a recently identified autoimmune disorder of the peripheral nerve myelin. Association of motor neuropathies or neuronopathies with thyroid disorders, such as hyperthyroidism, hypothyroidism or thyroid neoplasms has been rarely described. We studied a 61-year-old man with a 2-year-history of slowly progressive weakness of the left limbs with atrophy and fasciculations. Nerve conduction velocity studies revealed multifocal motor conduction blocks. Serum IgM titer of antibodies against GM1 was elevated (1:1280; n.v. up to 1:640). Thyroid studies were compatible with Hashimoto's thyroiditis. Therapy with high dose intravenous immunoglobulins was followed by a prompt clinical recovery. Then the disease assumed an intravenous immunoglobulins dependent course with a full clinical, but transient, recovery. This is the first observation of an association of multifocal motor neuropathy with high titers of GM1 and Hashimoto's thyroiditis and reinforces the multifocal motor neuropathy autoimmune origin as well as the repeated clinical recoveries after intravenous immunoglobulins. This case also suggests to deeply investigate the thyroid function in patients with multifocal motor neuropathy.     

Multifocal motor neuropathy, type 1 diabetes and asymptomatic Hashimoto's thyroiditis: an unusual association

Immune mediated mechanisms play a role in some forms of diabetic neuropathies. We studied a 17-year-old man who developed asymmetric weakness and atrophy in both upper arms soon after the diagnosis of type 1 diabetes. Nerve conduction studies demonstrated multifocal motor conduction blocks, and serological investigations revealed subclinical Hashimoto's thyroiditis. Therapy with intravenous immunoglobulin and cyclophosphamide led to clinical recovery. This is the first observation of an association between type 1 diabetes, multifocal motor neuropathy and Hashimoto's thyroiditis.     

Early neurophysiological evolution of chronic inflammatory demyelinating polyneuropathy in a patient with Hashimoto's thyroiditis

A patient with a known history of hypothyroidism due to Hashimoto's thyroiditis presented with a subacute, progressive sensorimotor deficit that affected the upper limbs predominantly. The electrophysiological findings progressively evolved from multifocal motor conduction block to multifocal demyelinating sensory and motor nerve involvement with conduction block, and finally to findings fulfilling the diagnostic criteria of chronic inflammatory demyelinating polyneuropathy (CIDP). The patient did not respond adequately to intravenous immunoglobulin, whereas oral prednisone led to fast and complete recovery. This report discusses the evolution of early findings of CIDP, as well as its coexistence with Hashimoto's thyroiditis.     

Epilepsia partialis continua in progressive multifocal leukoencephalopathy: a motor cortex isolation syndrome.

We describe the clinical and neuropathological features in a patient aged 45 years with progressive multifocal leukoencephalopathy with epilepsia partialis continua. The motor cortex and basal ganglia were preserved. Our findings lend support to the notion of isolation of the motor cortex as the cause of this particular type of focal status epilepticus.     

Hashimoto's encephalopathy. Case report and literature review

We present a 43-year-old man with recurrent episodes of Hashimoto's encephalopathy who was diagnosed with autoimmune thyroiditis in childhood. Encephalopathy started with subacute dementia followed by extrapyramidal and psychiatric symptoms of insidious onset. He had also status epilepticus which occurred within the first year of the disease. The patient was in euthyreosis, but increased levels of antithyroid antibodies were found. MRI of the brain was normal. Electroencephalography was initially normal and later showed diffuse slowing with generalized theta/delta activity. The cerebrospinal fluid examination revealed a high level of protein which decreased when remission of the disease was achieved. After other etiology was excluded Hashimoto's encephalopathy was diagnosed. Almost complete clinical recovery after steroid administration was observed. Attempts of prednisone withdrawal led to recurrence of neurological and psychiatric symptoms. The diagnosis of Hashimoto's encephalopathy should be considered in each case with subacute encephalopathy associated with high levels of antithyroid antibodies (despite normal thyroid function) and in the absence of other brain diseases.     

Can absence status epilepticus be of frontal lobe origin?

Five women with an unclassifiable nonconvulsive status epilepticus (NCSE) characterized by young age at onset, prolonged confusions, focal motor seizures, and both generalized spike-and-wave discharges and focal epileptic discharges on the EEG were studied with video-EEG monitoring. Electrographically, the NCSE originated from the left frontal lobe in 4 patients, and the left hemisphere with multifocal seizure discharges in 1 patient. Focal motor seizures seemed to originate from the left hemisphere in all 5 patients, particularly from its anterior part in 3 of them. Results show that the NCSE is complex partial status epilepticus of frontal lobe origin electroclinically mimicking absence status epilepticus once it reaches a full-blown phase.     

以癫痫持续状态起病的桥本脑病1例分析

目的 探讨以癫痫持续状态为首发表现的桥本脑病的临床特征。方法 回顾性分析1例以癫痫持续状态为首发表现的桥本脑病患者的临床资料。结果 临床呈癫痫持续状态,入院时神志不清,间断抽搐伴瞳孔散大,间歇期仍昏迷,余无明显局灶定位体征; 血气pH值6.807,乳酸24 mmol/L,头颅CT、MRI检查均未见异常,2次腰穿脑脊液常规、生化均正常,自身免疫性脑炎抗体8项均阴性,甲状腺功能检测提示轻度甲减,抗甲状腺球蛋白抗体和抗甲状腺过氧化物酶抗体升高,诊断桥本脑病,给予丙种球蛋白及甲强龙冲击治疗后病情迅速并明显好转,用药次日患者神志转清楚,癫痫持续状态得到有效控制。结论 桥本脑病临床以癫痫持续状态为首发者少见,故遇到不明原因的脑病患者,需注意甲状腺功能及相关抗体检查,以尽快明确诊断,尽早治疗,最大程度地改善其预后。     

Epilepsia partialis continua: semiology and differential diagnoses.

Epilepsia partialis continua (EPC) is a rare form of focal status epilepticus. It may have vascular, immune-mediated, neoplastic or metabolic-toxic causes. The origin of EPC has been linked with the motor cortex. This has been solidly supported by sophisticated electrophysiological studies. Here, a series of video sequences from patients with EPC (due to Rasmussen encephalitis, early-stage multiple sclerosis, and steroid responsive encephalopathy with autoimmune thyroiditis), and other cases with repetitive myoclonic jerks or movement disorders (myoclonic epilepsy associated with ragged-red fibers, Jacksonian march, myoclonic seizures in other types of frontal lobe or idiopathic generalized epilepsies, and different types of tremor) is presented. [Published with video sequences].     

Auditory seizures in autoimmune epilepsy: a case with anti‐thyroid antibodies

In its classic presentation, Hashimoto's encephalopathy is an acute‐subacute complex neuropsychiatric syndrome with cognitive impairment, hallucinations, myoclonus, tremor or ataxia, associated with elevated anti‐thyroid antibodies. Corticoids and immunotherapy are dramatically effective. However, in some cases, not all the associated features are presented and this delays diagnosis and appropriate treatment. We describe a man with abrupt onset of recurrent auditory seizures resulting in refractory non‐convulsive status epilepticus. The patient was diagnosed with an autoimmune encephalopathy with elevated serum and CSF anti‐thyroid antibodies. None of the antiepileptic drugs were successful, however, following immune‐modulating therapy, the refractory non‐convulsive status epilepticus dramatically improved, as did the patient overall. We suggest that Hashimoto's encephalopathy should be suspected in otherwise healthy patients with unexplained new‐onset focal recurrent auditory seizures which do not respond to antiepileptic drugs. The presence of anti‐thyroid antibodies in the CSF supports this diagnosis.     

Alien hand syndrome after epilepsia partialis continua: FDG PET and MRI studies

Epilepsia partialis continua (EPC) is clinically defined as a syndrome of continuous focal jerking of a body part, usually a distal limb, occurring over hours, days, or even years. It is considered the status epilepticus equivalent of simple partial motor seizures. A 48-year-old right-handed man with a history of traumatic intracranial hemorrhage was admitted for right-sided hemiplegia and drowsiness after complex partial status epilepticus. An EEG showed periodic lateralized epileptiform discharges over the left hemisphere. Brain MRI revealed extensive multifocal encephalomalaciac changes in the left temporo-parieto-occpital lobe and both frontal lobes with some hemorrhagic residual change. After administration of a loading dose of intravenous phenytoin, his mental status returned to normal. However, his weakness only partially improved. [(18)F]Fluorodeoxyglucose PET (FDG-PET) demonstrated severe hypometabolism in the left cerebral hemisphere, including the basal ganglia and thalamus, with cerebellar diaschisis. At the 3-month follow-up, he complained of symptoms of alien hand phenomenon. Follow-up MRI revealed more extensive encephalomalaciac changes in previously noted regions with thinning of the posterior end of the body of the corpus callosum. Moreover, FDG-PET demonstrated persistent severe hypometabolism over the left cerebral hemisphere. We suggest that the alien hand phenomenon was a result of thinning of the corpus callosum related to EPC.     

Steroid-responsive encephalopathy associated with autoimmune thyroiditis and primary CNS demyelination

Steroid responsive encephalopathy associated with autoimmune thyroiditis is a well-recognized complication of autoimmune thyroid disease. However, the characterization of the histopathological features of steroid responsive encephalopathy associated with autoimmune thyroiditis are limited to six cases. Reported pathological features include vasculitis involving venules and arterioles, lymphocytic perivascular cuffs and microglial activation. We report a case of SREAT with biopsy proven (on two occasions) primary CNS demyelination and radiological evidence of steroid responsiveness, identifying primary CNS demyelination as a complication of autoimmune thyroid disease.     

Hashimoto encephalopathy - is it underdiagnosed in pediatric patients?

Hashimoto encephalopathy (HE) is associated with Hashimoto thyroiditis. Clinically it presents with variable symptoms like seizures, neuropsychiatric changes or focal neurological deficits. Autoimmune phenomena are hypothesized for the pathogenesis. HE has mainly been described in the adult population. We present two 14-year-old patients who presented with recurrent seizures and mental decline. SPECT and PET scans showed distinctly pathological changes. Both patients were diagnosed with HE and improved dramatically on steroids. We feel that HE is a rare but important differential diagnosis of encephalopathy also in the pediatric population. As this disease responds well to steroids, we recommend to obtain basic thyroid function tests as well as thyroid antibodies in all cases of unexplained encephalopathy or unexplained status epilepticus.     

Multifocal motor neuropathy

PURPOSE OF THIS REVIEW: To conduct a critical review of recent studies on the clinical and therapeutic aspects of multifocal motor neuropathy, and to analyse their implications for patient management. RECENT FINDINGS: Recent studies have contributed to defining the specific position of multifocal motor neuropathy within the spectrum of chronic immune-mediated polyneuropathies. One study compared features of this condition with multifocal acquired demyelinating sensory and motor neuropathy, while others have focused on pathological alterations at the site of conduction blocks. A further study described six new cases of multifocal acquired motor neuropathy, which should be considered as a variant of multifocal motor neuropathy. Several Cochrane reviews and review articles have shown evidence of the efficacy of intravenous immunoglobulins in the treatment of multifocal motor neuropathy. The issue of long-term intravenous immunoglobulins in multifocal motor neuropathy, however, has yielded controversial results. Two studies have shown progressive motor deterioration in most patients, correlated with electrophysiological signs indicative of axonal degeneration, while a third study found signs of sustained clinical and electrophysiological improvement after a mean follow up of 7.25 years. SUMMARY: Multifocal motor neuropathy is a distinct clinical entity that differs from chronic inflammatory demyelinating polyradiculoneuropathy and multifocal acquired demyelinating sensory and motor neuropathy, although they share some electrophysiological characteristics. Although the aetiology remains unsolved, frequent association with high-titer antibodies against ganglioside GM1, together with an often positive response to intravenous immunoglobulins further support an autoimmune mechanism. New therapeutic strategies are required, however, that focus on the effects and the costs of treatment over long-term follow up.     

Hashimoto encephalopathy: literature review

Hashimoto encephalopathy (HE) presents as an encephalopathy without central nervous system infection or tumor. HE is associated with autoimmune thyroiditis and is thus considered to be an autoimmune disorder. The prevalence of HE is low, but death and status epilepticus have been reported. HE manifests with a wide range of symptoms that include behavioral changes and confusion. Elevated thyroid antibodies are present in the majority of cases and are required for the diagnosis of HE. Normal brain MRI findings are found in the majority of patients diagnosed with HE. The most consistent CSF abnormality noted in HE patients is the presence of elevated protein. Most HE patients respond well to steroid therapy. Clinical improvements are also observed with IV immunoglobulin and plasmapheresis. In conclusion, it is now generally accepted that the diagnosis of HE must include encephalopathy characterized by cognitive impairment associated with psychiatric features, such as hallucinations, delusions, and paranoia. Autoimmune encephalitis and prion disease should be considered in the differential diagnosis due to the similarity of the clinical features of these conditions to those of HE.     

Hashimoto's encephalopathy: toxic or autoimmune mechanism?]

A 36-year-old woman presented with partial complex status epilepticus. Magnetic resonance imaging with T2-weighted sequences showed a high-intensity signal in the left posterior frontal area. Hashimoto's thyroiditis was then discovered. The disappearance of the high-intensity signal after corticosteroid therapy was suggestive of an autoimmune mechanism. However, improvement could be obtained only with a hormonal treatment, which supports the hypothesis of a pathogenetic role of the Tyrosine-Releasing Hormone (TRH).     

Subacute cerebellar syndrome and Hashimoto's thyroiditis. Association or simple coincidence?

Numerous neurological symptoms have been observed in thyroid autoimmune disorders. The case of a 47-year-old euthyroid woman who develops a cerebellar syndrome associated with thyroiditis is discussed. Reference is made to the favorable outcome of combined per os thyroxine, corticosteroid and intravenous human normal immunoglobulin (Sandoglobulin) treatment.     

Intravenous sodium valproate in status epilepticus

A study was conducted to observe the effect of intravenous sodium valproate in status epilepticus. Eleven patients with status epilepticus, who were resistant to conventional drugs, underwent treatment with intravenous sodium valproate. The seizures were controlled in 10 patients within 24-48 hrs of starting treatment. No complications were observed during therapy. We conclude that intravenous sodium valproate can be recommended for Myoclonic status epilepticus and non-convulsive status epileticus.     

Encephalopathy associated to autoimmune thyroid disease: a more appropriate term for an underestimated condition?

Hashimoto's encephalopathy is a severe and rather infrequent clinical condition initially described in patients suffering from chronic lymphocytic thyroiditis. Its origin is still controversial but it can be agreed to have an autoimmune etiology. In fact, its most characteristic finding is the high titre of antithyroid antibodies, especially antimicrosomal. We describe three cases of Hashimoto's encephalopathy and establish a relationship between the clinical status, the antithyroid antibody levels and its response to corticosteroid treatment. There was an excellent response to corticosteroid treatment in all three cases. Interestingly, one case was associated with Graves' disease. Given this, and after the review of the literature, we believe that the term 'encephalopathy associated to autoimmune thyroid disease' could be more appropriate to define this entity. Finally, we suggest that autoimmune thyroid encephalopathy must be suspected in the face of unaccounted acute or subacute encephalopathy with high levels of antithyroid antibodies.     

Autoimmune thyroiditis and acquired demyelinating polyradiculoneuropathy

Guillain-Barrè syndrome (GBS) and Miller-Fisher syndrome (MFS) are variant forms of acquired demyelinating polyradiculoneuropathy. Their concurrence with immune disorders of the thyroid is infrequent. We report on a 7.5-year-old girl in whom a subclinical thyroiditis was concurrently detected to GBS and a 70-year-old woman with Hashimoto's thyroiditis (HT) who had recurrent MFS. Even though autoimmune thyroiditis is associated with many autoimmune disorders more often than would be expected by chance alone, its concurrence with immune disorders of the peripheral nerve is less frequently reported. The calculated coincidental concurrence of acquired demyelinating polyradiculoneuropathy (in both variants, MFS and GBS) and autoimmune thyroiditis (as in the present cases) was extremely low (0.0004%), thus suggesting common pathogenic mediators.     

Hashimoto's encephalopathy and motor neuron disease: a common autoimmune pathogenesis?

Hashimoto's encephalopathy is a rare complication of autoimmune thyroiditis not associated with thyroidal function decline. We report a 50-year-old man presenting with lower motor neuron symptoms evolving over 3 years and changes in behavior associated with attentive and cognitive impairment occurring in the last few months. Memory deficits, emotional instability, marked dysarthria, mild symmetric weakness of the lower extremities and fasciculations were the most striking clinical features. EEG was diffusely slow, cranial MRI revealed multiple subcortical white matter lesions, CSF protein was slightly elevated, electromyographic recordings showed acute and chronic denervation and extremely high TPO antibody titers were found in the serum. Hashimoto's encephalopathy and lower motor neuron disease were diagnosed. As repeated high-dose intravenous methylprednisolone administration followed by oral tapering improved both central nervous system and lower motor neuron symptoms, the question was raised whether there was a common autoimmune pathogenesis of both clinically distinct diseases.